Primäres zerebelläres T-Zell-Lymphom

Primary central nervous system T-cell lymphomas are rare and have to be differentiated from reactive lesions. It is therefore essential to use all possible tools to establish the diagnosis, including immunohistochemistry, molecular genetic analysis, and/or cytogenetic methods. In this paper we present the case of a primary cerebellar T-cell lymphoma in a 50-year-old man; a clonal T-cell receptor gene rearrangement was documented. After two cycles of methotrexate therapy the patient developed Pneumocystis carinii-induced pneumonia, dying 10 weeks after his diagnosis. The autopsy did not reveal any residual tumour.     

Primäres Sarkom des Pankreas

Ohne ZusammenfassungHierzu eine Abbildung im Text.     

Primäres Sarco-Carcinom der Pleura

Ohne ZusammenfassungHierzu Taf. V. Fig. 6–9.     

Primäres Synovialsarkom der Lunge

In the biopsy material of a polypoid intrabronchial lesion of a 54-year-old male patient with radiologically established lung tumor, spindle-cell neoplasia was seen. No clear histogenetic classification of the submucous tumor process was possible. After resection of the right upper lobe of the lung, including the results of additional immunohistochemical analyses, the tumor was classified as biphasic synovial sarcoma. This was suggested mostly by immunohistochemical detection of the epithelial membrane antigene (EMA) and of vimentin. Extensive clinical examinations ruled out the possibility of a primary tumor with extrapulmonary localization. This case report shows the problems faced in correctly classifying the histogenesis or the direction of differentiation in extremely rare cases of primary pulmonary synovial sarcoma. Especially in small biopsy samples showing a submucous spindle-cell tumor of the lung, apart from frequent sarcoma metastases, the presence of primary synovial sarcoma of the lung has to be considered in the differential diagnosis.     

Primäres Fibromyxom des linken Vorhofes

Ohne ZusammenfassungHierzu Taf. X.     

Primäres metastasirendes Sarcom der Milz

Ohne Zusammenfassung     

Primäres epitheloides Angiosarkom der proximalen Trachea

A 81-year-old man presented with massive hemoptysis and dyspnea. Results of chest radiography and computer tomography were highly suspicious for a large right-sided tumor of the bronchus with impression of the right main bronchus. Because of clinical worsening the patient was transferred to our department of pulmonology. The patient died of unappeasable hemoptysis the next day. At autopsy we found a 2-cm-diameter tumor of the proximal trachea as the source of massive bleeding and blood aspiration. Histological examination confirmed the tumor to be an epithelioid angiosarcoma of the trachea, which was a primary as no other tumor manifestation was detected. The entity of epithelioid angiosarcoma as a primary tumor of the trachea, to our knowledge, has not yet been reported.     

Primäres Cholangiokarzinom auf dem Boden einer Caroli-Erkrankung

Caroli's disease is a liver disease with segmental cystic dilatation of the intrahepatic bile ducts. It belongs to the group of congenital ductal plate malformations. With an incidence of only 0.05% of all liver cases in the Liver Registry of the University of Cologne, it is a very rare disorder. Caroli's disease is usually combined with cholangitis and bile duct stones. Control of these infections and maintenance of biliary drainage are the main therapeutic aims. The development of intra epithelial neoplasia and invasive carcinoma are rare complications. We report a case of Caroli's disease with the development of cholangiocarcinoma and review the literature.     

Primäres duktales Adenokarzinom im Bereich der Orbita

The case of a 59-year-old patient is described who presented with a left-sided pressure sensation in the left orbit and exophthalmus with a proud bulbus. The limited bulbus motility led to double vision. A preoperative MRI showed a space-occupying lesion in the left medial orbit. The morphological and immunohistochemical findings led to the diagnosis of a primary ductal carcinoma resembling salivary duct carcinoma (SDC). The tumor could be removed without compromising the eye. The patient suffers no side-effects or recurrences 12 months later.     

Primäres Osteosarkom der Leber Fallbericht und Literaturübersicht

Primary sarcomas of the liver are rare. Most of them are angiosarcomas often related to exposure to thorotrast or polyvinyl chloride. We report a case of primary osteosarcoma of the liver in a 72-year-old man and compare it with the seven cases from the literature     

Multiple Primärtumoren

Ohne ZusammenfassungMit 1 Abbildung im Text.     

Primäre angeborene Herzhypertrophie

Ohne Zusammenfassung     

Hereditäres Magenkarzinom

Hereditary diffuse gastric cancer is an autosomal dominant cancer syndrome with approximately 70%–80% penetrance. The cumulative lifetime risk of clinically detected gastric cancer is 63%–83% for women and 40%–67% for men. The average age at diagnosis is 40 years. Approximately 25%–40% of patients carry a germline mutation of the CDH1 gene. This gene encodes the transmembrane protein E-cadherin which plays a central role in cell adhesion and signal transduction. Classified according to Laurén, patients develop multifocal diffuse signet-ring cell carcinoma and, in late stages, linitis plastica. In the foveolar neck region, the site of gastric stem cells, in situ signet-ring cell carcinoma has been identified as a precursor lesion of invasive cancer. Therein, pagetoid spread of tumour cells below the preserved epithelium within the basal membrane represents the characteristic morphology. PAS staining may facilitate detection of tiny lesions.The present article provides detailed information on this cancer syndrome from the point of view of the pathologist as well as the human geneticist, focussing on the multidisciplinary management of affected patients.     

Primärer melanotischer Gehirntumor

Ohne ZusammenfassungHierzu Tafel IV.     

Spermatozytäres Seminom

Spermatocytic seminomas affect 0.3–0.8 per one million men. This tumor is not, as the name might suggest, a variant of classical seminoma but a distinct nosological entity, which differs markedly from all other germ cell tumors in its epidemiology, peculiar morphology, oncogenesis and clinical outcome. There are no racial differences in the incidence and risk factors are completely unknown. Patients are significantly older than is the case for other germ cell tumors with an average of 53.5 years; nevertheless, more than 25 % are younger than 40 years. Spermatocytic seminoma arises from differentiated spermatogonia, not from intratubular germ cell neoplasms. The tumor-specific gain of chromosome 9 seems to be the most important event in the oncogenesis. Conventional spermatocytic seminoma consists of three different cell types which give the tumor a highly aggressive appearance, although in actual fact, the tumor has a very favorable outcome, with few reported cases of general metastatic spread. Anaplastic spermatocytic seminoma, a recently described variant, also takes mostly a benign course; however, spermatocytic seminomas combined with sarcomas are extremely malignant.     

„Primäres” Achseldrüsencarcinom nach chronischer (carcinomatöser) Mastitis

Ohne Zusammenfassung     

Primäre Aktinomykose des Magens

Ohne ZusammenfassungMit 9 Textabbildungen.